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RESEARCH PRODUCT
IC3D Classification of Corneal Dystrophies—Edition 2
Kanwal K. NischalFrancis L. MunierChristopher J. RapuanoAntoine LabbéCecilie BredrupHans Ulrik MøllerShigeru KinoshitaEung Kweon KimJohn E. SutphinMassimo BusinAnthony J. AldaveTero KiveläBerthold SeitzKenneth R. KenyonWalter LischJayne S. Weisssubject
Macular corneal dystrophygenetic structuresEndothelial dystrophiesGenetic diseaseStromaEpitheliumGelatinousdrop-like corneal dystrophyCorneaLisch Epithelial Corneal DystrophyCornea pathologyPosteror polymorphous corneal dystrophyCorneal Dystrophies HereditaryPosterior amorphous corneal dystrophyEpithelial-stromal TGFBI dystrophiesMacular corneal dystrophyFleck corneal dystrophyLattice corneal dystrophyPre-Descemet corneal dystrophyCongenital stromal corneal dystrophySubepithelialmucinous corneal dystrophySchnyder corneal dystrophyThiel-Behnke corneal dystrophyPosterior polymorphous corneal dystrophyEpithelial and subepithelial dystrophiesFuchsendothelial corneal dystrophyFleck corneal dystrophyReis-Bücklers corneal dystrophyCongenital hereditary endothelial dystrophyCentralcloudy dystrophy of FrançoisCongenital stromal corneal dystrophyPosterior amorphous corneal dystrophymedicine.medical_specialtyHistologyeducationHereditary diseaseHistopathologyBiologyKeratoconusLisch epithelial corneal dystrophyMeesmann dystrophyNOBowman membraneDescemetmembraneInternational Classification of DiseasesTerminology as TopicOphthalmologyGeneticsmedicineHumansBowman membrane; Centralcloudy dystrophy of François; Confocal microscopy; Confocal microscopy; Congenital corneal endothelial dystrophy and X-linked endothelialdystrophy; Congenital stromal corneal dystrophy; Cornea; Cornea; Cornea dystrophy; Cornea pathology; Descemetmembrane; Endothelial dystrophies; Endothelium; Epithelial and subepithelial dystrophies; Epithelial basement membranedystrophy; Epithelial recurrent erosion dystrophies; Epithelial-stromal TGFBI dystrophies; Epithelium; Fleck corneal dystrophy; Fuchsendothelial corneal dystrophy; Gelatinousdrop-like corneal dystrophy; Genetic disease; Genetics; Granular corneal dystrophy type 1; Granular corneal dystrophy type 2; Hereditary disease; Histology; Histopathology; Keratoconus; Lattice corneal dystrophy; Lisch epithelial corneal dystrophy; Macular corneal dystrophy; Meesmann dystrophy; Posterior amorphous corneal dystrophy; Posteror polymorphous corneal dystrophy; Pre-Descemet corneal dystrophy; Reis-Bücklers corneal dystrophy; Schnyder corneal dystrophy; Stroma; Stromal dystrophies; Subepithelialmucinous corneal dystrophy; TGFBI; Thiel-Behnke corneal dystrophy; OphthalmologyEndotheliumEpithelial basement membranedystrophyCornea dystrophyCongenital corneal endothelial dystrophy and X-linked endothelialdystrophymedicine.diseaseeye diseasesConfocal microscopyOphthalmologyGranular corneal dystrophy type 2Granular corneal dystrophy type 1Stromal dystrophiesLattice corneal dystrophysense organsTGFBIEpithelial recurrent erosion dystrophiesdescription
To update the 2008 International Classification of Corneal Dystrophies (IC3D) incorporating new clinical, histopathologic, and genetic information.The IC3D reviewed worldwide peer-reviewed articles for new information on corneal dystrophies published between 2008 and 2014. Using this information, corneal dystrophy templates and anatomic classification were updated. New clinical, histopathologic, and confocal photographs were added.On the basis of revisiting the cellular origin of corneal dystrophy, a modified anatomic classification is proposed consisting of (1) epithelial and subepithelial dystrophies, (2) epithelial-stromal TGFBI dystrophies, (3) stromal dystrophies, and (4) endothelial dystrophies. Most of the dystrophy templates are updated. The entity "Epithelial recurrent erosion dystrophies" actually includes a number of potentially distinct epithelial dystrophies (Franceschetti corneal dystrophy, Dystrophia Smolandiensis, and Dystrophia Helsinglandica) but must be differentiated from dystrophies such as TGFBI-induced dystrophies, which are also often associated with recurrent epithelial erosions. The chromosome locus of Thiel-Behnke corneal dystrophy is only located on 5q31. The entity previously designated as a variant of Thiel-Behnke corneal dystrophy on chromosome 10q24 may represent a novel corneal dystrophy. Congenital hereditary endothelial dystrophy (CHED, formerly CHED2) is most likely only an autosomal recessive disorder. The so-called autosomal dominant inherited CHED (formerly CHED1) is insufficiently distinct to continue to be considered a unique corneal dystrophy. On review of almost all of the published cases, the description appeared most similar to a type of posterior polymorphous corneal dystrophy linked to the same chromosome 20 locus (PPCD1). Confocal microscopy also has emerged as a helpful tool to reveal in vivo features of several corneal dystrophies that previously required histopathologic examination to definitively diagnose.This revision of the IC3D classification includes an updated anatomic classification of corneal dystrophies more accurately classifying TGFBI dystrophies that affect multiple layers rather than are confined to one corneal layer. Typical histopathologic and confocal images have been added to the corneal dystrophy templates.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2015-02-01 | Cornea |